[Jugal swelling revealing acute lymphoblastic leukemia]

Acute lymphoblastic leukemia (ALL) is the most common hematologic malignancy in children. The clinical presentation at diagnosis is due to bone marrow infiltration or extra-medullary involvement. Maxillofacial localization is very rare in ALL. We report a case of T-ALL revealed by right cheek swelling in a 14-year-old boy.

A 14-year-old boy had presented with a 6 cm right cheek swelling for two months, complicated by pyrexia, cervical adenopathy, and splenomegaly. CT scan revealed a 3×7×8 cm swelling surrounded by voluminous sub chin, subclavicular, mediastinal, and paratracheal bilateral adenopathies. The biological analyses revealed normocytic anemia, hyperleukocytosis with 83.6% blast cells, neutropenia, and thrombopenia. The biopsy histology and the immuno-histochemical analysis suggested a diffuse small-cell Non-Hodgkin lymphoma (NHL). The myelogram identified a type 1 ALL and immunophenotyping on bone marrow cells suggested phenotype T ALL. The patient was treated according to the MARALL-06 protocol and died on the 17th day of induction, in septic shock.

Despite their rare occurrence, ALL should be included in the differential diagnosis of jugal swelling. The evident hematological context should suggest the diagnosis and a myelogram should be performed as soon as possible because the outcome is rapidly fatal.